We report a case of rhabdomyosarcoma of the middle ear and mastoid in a 3-year-old boy. The patient was treated according to Intergroup Rhabdomyosarcoma Study IV protocol (chemo- and radiotherapy), and he experienced a complete remission. However, 7 months after the completion of treatment, he experienced a recurrence at the primary site that spread to the brain. Despite treatment, the patient died of progressive metastasis to the lung 4 months later.
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